Purpose:
In human and mouse, Aryl hydrocarbon receptor interacting protein-like 1 (AIPL1) is expressed in photoreceptors and required for stability and membrane anchoring of a phototransduction molecule, cGMP-phosphodiesterase 6 (PDE6). Genetic mutations of AIPL1 are linked to Leber congenital amaurosis 4 (LCA4) in human. Since genetic mutations of PDE6 also cause photoreceptor degeneration, chronically elevated levels of cGMP is toxic to photoreceptors. Purpose of this study is to understand molecular mechanism underlying photoreceptor cell death in response to dysfunctions of AIPL1/PDE6.